Acute rheumatic fever (ARF) continues to cause a large burden of mortality and morbidity in low- and middle-income countries. It is less common in high-income countries, but continues to be seen in indigenous communities and during outbreaks.
Currently, no single test can diagnose ARF. Diagnosis is clinical and relies on the Jones criteria.
The 5 major manifestations of ARF are carditis, arthritis, chorea, erythema marginatum, and subcutaneous nodules, of which the most common are carditis and arthritis.
The Jones criteria were revised in 2015 to include separate criteria for low-risk and moderate- to high-risk populations and introduce the use of Doppler echocardiography to diagnose subclinical carditis.
While all other manifestations of ARF resolve without sequelae, carditis can lead to chronic rheumatic heart disease (RHD).
Secondary prophylaxis is thought to ameliorate the progression of ARF to chronic RHD, but even the mildest forms of carditis can result in chronic RHD and decreased survival.
The prognosis of established rheumatic valvular disease remains guarded, especially with severe disease.
Acute rheumatic fever (ARF) is an autoimmune disease that mostly occurs following group A streptococcal throat infection, although there is growing evidence of ARF following skin infection in certain regions of the world. It can affect multiple systems, including the joints, heart, brain, and skin. Only the effects on the heart can lead to permanent illness; chronic changes to the heart valves are referred to as chronic rheumatic heart disease (RHD). Without long-term penicillin secondary prophylaxis, ARF can recur, leading to cumulative damage to the cardiac valvular tissue.
History and exam
Key diagnostic factors
- joint pain
Other diagnostic factors
- recent sore throat or scarlet fever
- recent skin infection
- chest pain
- shortness of breath
- heart murmur
- pericardial rub
- signs of cardiac failure
- swollen joints
- emotional lability and personality changes
- jerky, uncoordinated choreiform movements
- inability to maintain protrusion of the tongue
- milkmaid's grip
- spooning sign
- pronator sign
- erythema marginatum
- subcutaneous nodules
- pregnancy or taking oral contraceptive pill
- overcrowded living quarters
- family history of rheumatic fever
- HLA association
- genetic susceptibility
- indigenous populations; Aboriginal Australian, Asian, and Pacific Islanders
- D8/17 B cell antigen positivity
1st investigations to order
- erythrocyte sedimentation rate (ESR)
- WBC count
- blood cultures
- chest x-ray
- throat culture
- rapid antigen test for group A streptococci
- anti-streptococcal serology
- rapid molecular test
monoarthritis in unconfirmed rheumatic fever
possible rheumatic fever
confirmed rheumatic fever
all patients following acute treatment
- Septic arthritis
- Juvenile arthritis
- Post-infectious reactive arthropathy
- Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease
- Group A streptococcal infections: guidance and data
Rheumatic feverMore Patient leaflets
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